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Flashcards in Immunology Deck (61):
1

Hallmark of immune deficiencies

SPUR -
S - serious infections (unresponsive to oral antibiotics)
P - persistent infections (early structural damage, chronic infections)
U - unusual infections (unusual organisms or sites)
R - recurrent infections (2 minor or 1 major a year)

2

Other features of immune deficiency

Weight loss or failure to thrive
Severe skin rash (eczema)?
Chronic diarrhoea
Mouth ulcers
Unusual autoimmune disease
Family history

3

2 classifications of immunodeficiencies

Primary (rare)
Secondary (common)

4

2 branches of the immune system

Innate
Acquired

5

Cells in the innate immune system

Macrophages
Neutrophils
Mast cells
NK cells

6

Proteins of innate immune system

Complement
Acute phase protein
Cytokines

7

Function of innate immune system

Rapid clearance of microorganisms
Stimulates acquired immune response
Buys time for acquired immune response

8

Cells in the acquired immune system

B lymphocytes
T lymphocytes

9

Proteins in the acquired immune system

Antibody

10

Function of acquired immune system

Adaptive response after exposure to antigen
Responsive to unlimited number of molecules
Immunological memory

11

Examples of phagocytes

Neutrophils
Monocytes/Macrophages

12

Functions of phagocytes

Initiation and amplification of inflammatory response
Scavenge for cellular and infectious debris
Produce inflammatory molecules which regulate other parts of IS
Resolution and repair

13

Clinical features of phagocytes deficiencies

Recurrent infections
May affect common or unusual sites
All types of bacteria and fungi

14

Where are phagocytes found

Bone marrow or within tissues

15

What is Kastmann syndrome?

Rare autosomal recessive disorder
Severe chronic neutropenia

16

Clinical presentation of Kastmann syndrome

Infections (0 - 2 weeks old)
Fever
Irritability
Oral ulcers
Failure to thrive

17

Treatment of Kastmanns syndrome

Supportive
- prophylaxis antibiotics & antifungals
Definitive
- stem cell transplant
G-CSF

18

What is leukocyte adhesion deficiency?

Rare primary immunodeficiency
Caused by genetic defect
Results in failure of neutrophil adhesions and migration

19

Clinical features of leukocyte adhesion deficiency

Marked leukocytos
Localised infections - difficult to detect

20

What causes chronic granulomatous disease?

Failure of oxidative killing mechanisms

21

What are the features of chronic granulomatous disease?

Recurrent deep bacterial infections
Recurrent fungal infections
Failure to thrive
Lymphadenopathy & hepatosplenomegaly
Granuloma formation

22

Investigation for chronic granulomatous disease

NBT test

23

Treatment of chronic granulomatous disease

Supportive
- prophylactic antibiotics/anti-fungals
Definitive
- stem cell,transplant
- gene therapy

24

Why are intracellular organisms difficult for the immune system?

Hide from immune system by locating within cells
Can hide in immune cells (esp. macrophages)
Need specific strategies to clear infection

25

Examples of intracellular organisms

Salmonella
Chlamydia
Rickettsia

26

What network defends against intracellular organisms?

IL12- gIFN network

27

Brief summary of IL12-gIFN network

Infected macrophages stimulated to produce Il12
IL12 induces T cells to produce gIFN
gIFN feeds back to macrophage and neutrophils
Stimulate TNF production
Activate NADPH
Stimulates oxidative pathways

28

Causes of defects in IL12-gIFN network

Single gene defects
- gIFN receptor deficiency
- IL12 deficiency
- IL12 receptor deficiency

29

Treatment of phagocyte deficiencies

Supportive
- prophylaxis
- oral/IV antibiotics
- surgical drain of abscess
Definitive
- bone marrow transplant
- gIFN therapy
- gene therapy

30

4 organisms that the immune system tackles

Viruses (e.g. Influenza)
Bacteria (e.g. Staph/Strep)
Protozoa (e.g. Amoeba)
Worms (e.g. Guinea worm)

31

Where do lymphocytes mature?

Thymus gland

32

Where is the thymus gland?

Just in front of the heart in the mediastinum

33

How is lymph returned to the systemic circulation?

Thoracic duct

34

What does the thoracic duct join with?

Left subclavian vein

35

What does IL-1 do?

Causes fever and activates lymphocytes

36

What does Il- 6 do?

Causes fever, stimulates the liver to produce acute phase proteins such as CRP, activates lymphocytes and promotors antibody production

37

What does IL-8 do?

Causes neutrophil Chemotaxis

38

What does IL-12 do?

Activates NK cells and TH1 cells (important for intracellular infections)

39

What does TNF-alpha do?

Increases vascular permeability to allow immune cells to reach tissues

40

What do IL-4, IL-5 and IL-13 do?m

Promote IgE production and eosinophilic reactions inpatients with allergies

41

What does gamma interferon do?

Activates cell mediated immunity in viral infections

42

What does IL-10 do?

Has an anti inflammatory affect

43

What do co stimulators molecules do?

Encourage activation of the adaptive immune system

44

How are dendritic cells involved in antigen presentation?

Carry digested antigens to lymph nodes
Present to TH0 cells with MHC II complexes

45

What can TH0 cells differentiate into?

TH1 and TH2

46

How are hypersensitivity reactions classed?

Type I: immediate hypersensitivity
Type II: direct cell killing
Type III: Immune complex mediated
Type IV: delayed type hypersensitivity

47

Summary of the path ophthalmology of allergic reactions

Production of specific IgE directed against antigen
IgE allergen complex binds to mast cells via Fc receptors
De granulation of mast cells
Release of preformed and newly synthesised inflammatory molecules (e.g. histamine, leukotrienes)

48

What is sodium cromoglycate?

Mast cell stabiliser

49

Mechanism of action of anti-histamines

H1 receptor antagonists
Block affects of histamines

50

Example of leukotriene receptor antagonist

Montelukast

51

Action of adrenalin in anaphylaxis

Acts on B2 adrenergic receptors to constrict arterial smooth muscle
Increases blood pressure limiting vascular leakage
Dilates bronchial smooth muscle

52

Key feature of Type II hypersitivity reaction

Antibody binds to cell surface antigen

53

4 actions of complement

Chemotaxis (recruits neutrophils and macrophages)
Solubilisation (of immune complexes)
Direct killing (of encapsulated bacteria - MAC punches hole)
Opsonisation (tomato ketchup)

54

Clinical example of type II hypersensitivity reaction

Blood donor rejection

55

What is plasmapheresis?

Cell separator to remove pathogenic antibody

56

Example of type III hypersensitivity reaction

Farmers lung
Pigeon fanciers lung
Systemic lupus erythematosus (SLE)

57

Examples of type IV hypersensitivity

Nickel hypersensitivity

58

Management of sarcoidosis

NSAIDS (for acute onset)
Systemic corticosteroids
- block T cell activation
- block macrophage activation

59

Diseases characterised by type IV hypersensitivity and granuloma formation

Sarcoidosis
Tuberculosis
Leprosy (some forms)
Berylliosis, silicosis and other dust diseases
Chronic stage of hypersensitivity pneumonitisis

60

Types of transplant rejection

Hyperacute rejection
Acute cellular rejection
Acute vascular rejection
Chronic allograft failure

61

Treatment for transplant rejection

Immunosuppressive